Surgical management of pain or infection secondary to calciphylaxis: a wound center's experience and algorithmic multidisciplinary approach.

BACKGROUND: The role of surgical management of calciphylaxis remains understudied. OBJECTIVE: This article reports a case series and algorithmic approach to the multidisciplinary management of calciphylaxis. METHODS: A single-center retrospective review of all adult patients with calciphylaxis treated surgically between January 2010 and November 2022 was performed. RESULTS: Eleven patients met inclusion criteria. The average age was 50.9 years ± 15.8 SD, and most patients were female (n = 7 [63.6%]). Surgery was indicated for infection (n = 6 [54.5%]) and/or intractable pain (n = 11 [100%]). Patients underwent an average of 2.9 excisional debridements during their hospital course. Following the final excision, wounds were left open in 5 cases (29.4%), closed primarily in 4 (23.5%), and local flaps were used in 3 (27.3%). Postoperatively, the mean time to healing was 57.4 days ± 12.6. Complications included dehiscence (n = 1 [9.1%]), progression to cellulitis (n = 2 [18.2%]), osteomyelitis (n = 1 [9.1%]), and lower extremity amputation (n = 2 [18.2%]). Of the 6 patients alive at the time of healing, 5 (83.3%) were no longer taking narcotic medications. At an average follow-up of 26.4 months ± 34.1, 7 patients (63.6%) were deceased, with an average time to mortality of 4.8 months ± 6.7. Of the 4 remaining patients, 3 (75.0%) were ambulatory by their most recent follow-up visit. CONCLUSION: While the morbidity and mortality associated with calciphylaxis are substantial, surgical excision is effective in reducing pain and improving quality of life in patients with this end-stage disease. Wound care centers are uniquely equipped with a variety of medical and surgical specialists with experience in treating chronic wounds and thus facilitate an efficient multidisciplinary model.

[A Case of Penile Calciphylaxis in a Hemodialysis Patient].

In the present case of a 56-year-old male, hemodialysis was introduced from December 20XX-2 due to chronic renal failure caused by diabetic nephropathy. In February 20XX, a glans penis ulcer was observed. It gradually expanded. Angiography conducted in April revealed complete occlusion of the left internal pudendal artery and poor visualization of the bilateral penile arteries. Given the high risk of obstruction, endovascular treatment was not conducted. The glans penis ulcer continued to expand, and maintenance dialysis became difficult due to intractable pain. Opioids were introduced, but the pain could not be controlled. In May 20XX, the patient was referred to our department for surgical treatment, and partial penile resection was performed. The patient was diagnosed with penile calciphylaxis based on clinical findings and pathological diagnosis. After the surgery, the pain subsided considerably, and the patient is being followed on an out-patient basis.

Successful Treatment of Calciphylaxis in a Young Female With End-Stage Renal Disease on Peritoneal Dialysis With Parathyroidectomy, Intensification of Dialysis, and Sodium Thiosulphate-A Case Report and Literature Review.

Calcific uremic arteriolopathy, commonly referred to as "calciphylaxis," is a rare life-threatening condition observed in patients with chronic kidney disease and end-stage renal disease on dialysis. This results in necrotic, ischemic, tender dermal lesions anywhere in the body, but mainly on the abdominal wall and lower extremities, where subcutaneous tissue is abundant. Histologically, it is defined by calcification in dermal capillaries, arterioles, and subcutaneous adipose tissues. It can occur in all advanced stages of chronic kidney disease as well as end-stage renal disease patients on hemodialysis or peritoneal dialysis. Our case highlights a successful case of calciphylaxis in a young female patient who underwent parathyroidectomy and intensification of peritoneal dialysis regimen along with the infusion of sodium thiosulphate injection resulting in complete resolution of the lesion in 3 months. With limited evidence of treatment options and increased frequency of this condition in a dialysis patient, our case highlights the key aspects of calciphylaxis management in a young end-stage renal disease patient who didn't need a change of dialysis modality. We also review the risk factors and current practiced management options of this condition in our article.

Multidisciplinary management of calciphylaxis: a series of 5 patients at a single facility.

Calciphylaxis is a rare and severe disease that manifests with painful skin ulceration and necrosis. Herein, we report five patients of hemodialysis patients with skin biopsy-proven calciphylaxis at a single facility. One patient had undergone parathyroidectomy (PTx) due to severe secondary hyperparathyroidism, four had been treated with vitamin D receptor activators, and two were on warfarin therapy. All patients had hyperphosphatemia, and one had hypercalcemia. The intact parathyroid hormone level at diagnosis was 2 pg/ml in the patient after PTx, while three patients were within the target range. The average period after diagnosis of calciphylaxis was 2 months. Skin lesions were present on the thighs and lower legs in two patients, and on the dorsum of the foot in one patient. In skin biopsy, calcification was found in the arteriolar media in four patients, and calcium (Ca) was deposited in the dermal lesion in one patient. All patients received local cures, surgical debridement, antibiotics to control infectious diseases, and strict control of serum Ca and phosphate. Calcimimetics were used in all patients except one who had undergone PTx one month before, sodium thiosulfate was used in 4 patients, and low Ca dialysate was used in three patients. The average follow-up period was 7.4 months. Four patients were cured, and one died due to infection. We suggest that multidisciplinary management for infectious diseases, surgical debridement, strict control of mineral and bone markers from the early stage, and elimination of risk factors may improve the course of calciphylaxis, which is a life-threatening disease.

Penile gangrene due to calciphylaxis: a multidisciplinary approach to a complex clinical challenge.

Penile calciphylaxis is a rare but clinically challenging condition to manage. While multiple treatment options have been proposed, the underlying evidence is anecdotal, and the overall prognosis remains extremely poor. We describe the case of a patient who underwent urgent debridement of a suspected gangrenous penile tip, who was intraoperatively found to have penile calciphylaxis. The necrosis progressed and the patient had a glans amputation. A non-healing wound developed, requiring reconstruction with two skin grafts using foreskin by the plastic and reconstructive surgical unit. The patient was commenced on sodium thiosulfate and his wound had completely healed 2 months following his last operation. We review the literature to determine the biochemical features, management options and outcomes in patients with penile calciphylaxis.

Parathyroidectomy as a Cure for Calciphylaxis in a Non-Dialysis Chronic Kidney Disease Patient?

BACKGROUND Calciphylaxis is a rare and fatal systemic disease which most commonly occurs in patients with end-stage renal disease. It is a poorly understood vascular calcification with unclear pathology that leads to vascular compromise due to vascular occlusion with endoluminal calcification. CASE REPORT We report a case of a 61-year-old male with chronic kidney disease stage 5 who developed calciphylaxis. The patient was diagnosed with dry gangrene of the second and third digits of the right hand and second, third, and fourth phalanges of the left hand. Despite medical therapy and local wound care, the lesions progressively worsened with time. The patient was found to have secondary hyperparathyroidism (parathyroid hormone was 1028 pg/mL) and underwent subtotal parathyroidectomy. In our patient, the skin lesions due to calciphylaxis completely resolved over the course of 12 months. CONCLUSIONS Parathyroidectomy has been associated with clinical benefit in patients with calciphylaxis. Clinicians should consider parathyroidectomy in the setting of high parathyroid hormone and calciphylaxis. Although parathyroidectomy is an effective treatment option for calciphylaxis it is not a definitive treatment and calciphylaxis can occur, though rarely, even after parathyroidectomy. There is a need to do further studies in order to confirm the efficacy of parathyroidectomy.

Removing the problem: parathyroidectomy for calciphylaxis.

Calcific uremic arteriolopathy (CUA), widely known as calciphylaxis, is a rare and lethal disease that usually affects patients with end-stage renal disease. It is characterised by widespread vascular calcification leading to tissue ischaemia and necrosis and formation of characteristic skin lesions with black eschar. Treatment options include sodium thiosulfate, cinacalcet, phosphate binders and in resistant cases, parathyroidectomy. We report a case of recurrent, treatment-resistant CUA successfully treated with parathyroidectomy. Her postoperative course was complicated by hungry bone syndrome and worsening of her wounds before they completely healed. We then discuss the morbidity of CUA, including the controversy around the use of parathyroidectomy and risk of aggressive management of hungry bone syndrome.

We Do Too Many Parathyroidectomies for Calciphylaxis.

Calciphylaxis, a disorder of dermal arteriolar calcification with a distinct predilection for patients with advanced kidney disease, remains an enigmatic condition that challenges clinicians. Observations regarding positive associations between levels of circulating parathyroid hormone (PTH) and the risk of calciphylaxis, combined with experimental data suggesting a pathogenic role of secondary hyperparathyroidism in vascular calcification, have led to the assumption that reducing PTH levels will improve the outcomes in patients with calciphylaxis. This editorial communicates the contemporary data regarding surgical parathyroidectomy as a treatment option for these patients. We raise concerns regarding the quality of available data and discuss the direction of future comparative effectiveness research in this field.

Successful Surgical Treatment of Severe Calciphylaxis Using a Bilayer Dermal Replacement Matrix.

Cutaneous calciphylaxis is a rare and often intractable disease that involves subcutaneous vascular calcification, ischemia, and subsequent necrosis. Calciphylaxis has an associated 60%-80% mortality rate with sepsis as the leading cause of death. However, despite variable success rates, the proper treatment of calciphylaxis remains controversial. In this case report, the authors present a 42-year-old female who presented with bilateral lower extremity calciphylaxis in conjunction with long-standing liver disease and acute renal failure. Cure of the patient's calciphylaxis was achieved through a surgical approach using staged debridement, placement of a dermal regenerative template (Integra Dermal Regeneration Template, Integra Lifesciences, Plainsboro, NJ), and followed by successful skin grafting. This is the first successful treatment of calciphylaxis in the literature to date using dermal regenerative template material.

Calcinosis Cutis and Calciphylaxis.

Calcinosis cutis (CC) is defined as the deposition of calcium salts in the skin. The condition is divided into 5 types: calciphylaxis and dystrophic, metastatic, idiopathic, and iatrogenic CC. Dystrophic CC is the most common form and usually occurs in association with autoimmune diseases. CC can be treated surgically or with the use of drugs such as diltiazem, bisphosphonates, warfarin, ceftriaxone, probenecid, minocycline, or aluminum hydroxide. Calciphylaxis is defined as calcification of the media of small- and medium-sized blood vessels in the dermis and subcutaneous tissue. Clinically, calciphylaxis causes livedo racemosa, which progresses to retiform purpura and skin necrosis. First-line treatment is with sodium thiosulfate. We present a review of the calcifying disorders of the skin, focusing on their diagnosis and treatment.

[Massive panniculectomy and bilateral subtotal mastectomy in a case of calciphylaxis: A case report and up date]. / Dermolipectomie abdominale et mastectomie bilatérale subtotale dans le cadre d'une calciphylaxie: à propos d'un cas et mise au point.

Calciphylaxis or calcific arteriolopathy is a rare, life-threatening obstructive pathology of the small cutaneous and subcutaneous vessels. It mainly affects patients with chronic renal failure but it also has been described in patients with normal renal function. The principal risks factors apart from renal failure and phosphocalcic metabolism imbalance are: the female sex, obesity, peripheral vascular disease, diabetes and oral anti-coagulation. We present a very rare case of abdominal, mammarian and upper thighs calciphylaxis in a patient with normal renal function. She presented a severe obesity with a recent important loss of weight and had been treated by oral anticoagulants for a long time. She benefited of a multidisciplinary approach with dermatologists, plastic surgeons and anesthesists permitting a recovery in fourteen weeks. Multidisciplinary approach is necessary but the place of the surgery is not well defined. We report a case in which early and wide surgical approach permitted to obtain a favourable evolution of the pathology. Then, we propose a therapeutic strategy after review of the literature.

Calciphylaxis and subtotal parathyroidectomy: a double-edged sword.

Calciphylaxis, also called calcific uremic arteriolopathy is a dreadful, life-threatening ischemic vasculopathy, primarily involving skin and subcutaneous tissue, mostly on patients with end-stage kidney disease. Calciphylaxis is a well-described, but still a poorly understood disorder of mineral metabolism. Its occurrence is rare, but increasingly reported. We describe a 62-year-old obese lady on hemodialysis for end-stage kidney disease due to type 2 diabetes, who developed two episodes of calciphylactic skin lesions during the 3 years observation. The healing of lesions in the first episode was prompted by a subtotal parathyroidectomy for severe secondary hyperparathyroidism. However, the resumption of oral calcium and vitamin D analog for the parathyroidectomy-related hypocalcemia induced another occurrence of widespread calciphylactic skin lesions in areas of great adiposity. The typical regimen of IV sodium thiosulfate brought a successful outcome to the second episode. Now, growing numbers of successful treatment for calciphylaxis are reported with more specific therapies and a clear understanding of the pathogenetic mechanism is in sight.

Calcific uremic arteriolopathy in hemodialyzed patients.

In the present article, we discuss the case of 67-year-old female patient diagnosed with inferior limbs calciphylaxis and hemodialyzed since 2006. The clinical manifestations and pathological lab findings are typical for this rare and extremely severe complication in chronic hemodialyzed individuals. The favorable treatment response to sodium thiosulfate, not often used as elected therapy in international studies, represents the particularity of the case.

Revascularization surgery for penile calciphylaxis.

Calciphylaxis, a systemic disorder seen in 1%-4% of patients with end-stage renal disease, is a cause of penile ischemic gangrene. We present a case of successful revascularization surgery for penile calciphylaxis. An arterial bypass to the deep dorsal penile vein relieved the rest pain and stopped expansion of the gangrenous lesion.

Complete resolution of calciphylaxis after kidney transplantation.

Calciphylaxis, a life-threatening and disabling complication in patients with end-stage renal disease, occurs most frequently in those treated with maintenance dialysis, whether it be hemodialysis or peritoneal dialysis. The impact of kidney transplantation on calciphylaxis lesions is not clear. The general consensus is to treat calciphylaxis adequately prior to transplantation with either medical therapy or parathyroidectomy, as indicated. We describe the case of a patient on peritoneal dialysis therapy who had severe calciphylaxis lesions that failed to resolve upon pretransplantation medical treatment and that then resolved after kidney transplantation.

Calciphylaxis: diagnosis and clinical features.

Calciphylaxis is a relatively rare disease, observed mainly in patients on dialysis, associated with high mortality rates, and characterized by painful skin ulceration. The pathogenesis of calciphylaxis is virtually unknown, although several risk factors, including warfarin therapy, hypoalbuminemia, and disturbances in calcium-phosphate metabolism, have been reported. The prevalence of calciphylaxis in Japan is likely to be less than 1:10,000 dialysis patients per year based on our nationwide survey in 2009. However, the results of the survey also showed that about 60 % of nephrologists in Japan are not familiar with the disease itself and it is highly likely that calciphylaxis is being overlooked. To facilitate recognition of calciphylaxis, we have proposed diagnostic criteria. At present, there is no specific therapy for calciphylaxis and general supportive measures, especially antibiotics for the accompanying infection and wound care, are important. Recently, sodium thiosulfate has been increasingly used to treat calciphylaxis and its efficacy should be evaluated by large clinical trials.

Calciphylaxis - a challenging & solvable task for plastic surgery? A case report.

BACKGROUND: Calciphylaxis (calcific uremic arteriolopathy) is rare and its pathogenesis is not fully understood. Indeed, Calciphylaxis presents a challenge through the course of its management which involve different specialities but unfortunately this disease so far has a poor prognosis. We herein present, in this case report, a multidisciplinary approach involving plastic surgeons with special regards to reconstructive approach after debridement procedures. CASE PRESENTATION: We present a 21 years old male with a BMI of 38,2, who was transferred to our department from another hospital. Calciphylaxis has been diagnosed after receiving anticoagulation with phenprocoumon after a single event of pulmonary embolism. The INR on admission was 1,79. He had necrotic spots on both sides of the abdominal wall and on both thighs medially. During this time he underwent several reconstructive procedures in our department. CONCLUSION: It can be suggested that this agonizing disease needs indeed a multidisciplinary approach involving Nephrologists, Dermatologists, Intensive Care Physicians and Plastic Surgeons, taking into consideration that surgical correction can achieve further improvement in a specialized centre. Notwithstanding, further cohort studies should be approached clinically to insight the light on this disease with special regard to the prognosis after this approach.

[Surgical approach to atypical wounds (clinical cases). Subcutaneous ischemic arteriolosclerosis (Martorell ulcer, calciphylaxis, eutrophication)]. / Kirurski pristup atipicnim ranama (klinicki primjeri) Supkutana ishemicka arterioloskleroza (Martorellov vrijed, kalcifilaksija, eutrofikacija).

Atypical wounds are probably the most delicate modern medicine topics as well as the most demanding surgical issue. Recently, we submitted an original report of two similar atypical vascular cases at our surgery department. Both presented a rare type of atypical, potentially fatal, vascular illness due to acute ischemic subcutaneous arteriolosclerosis. Because of the strikingly similar common pathophysiological features, Martorell hypertensive ischemic leg ulcer (HYTILU) and calciphylaxis require identical approach and therapy, both systemic and surgical. Even an experienced clinician can easily confuse it with other atypical wounds, namely pyoderma gangrenosum, which due to the corticosteroid induced immunodeficiency can be detrimental, since the two different approach strategies are required. Based on typical localization, necrotic painful skin necroses, progressive local deterioration, often difficult secondary infections along with long term hypertension and diabetes history could elucidate suspicion of ischemic subcutaneous arteriosclerosis. Hypertension (and often diabetes), local findings and histologically proven subcutaneous arteriolosclerosis are mandatory to make the diagnosis. Rapid local amelioration following correct treatment approach additionally confirms the presumed diagnosis. Besides the minutely repetitive surgical debridement, negative wound pressure therapy and split skin transplantation, one should consider systemic medication (analgesics, antioxidants, LMWH, sodium thiosulfate and antibiotics). Considering the cases presented, opportune decisions along with moderate aggressive and modern holistic surgical approach should inevitably resolve hard to heal atypical wounds.

Penile calciphylaxis: a life-threatening condition successfully treated with sodium thiosulfate.

Calciphylaxis or calcific uremic arteriolopathy is a life-threatening condition that predominantly affects patients with end-stage renal disease on hemodialysis. A prevalence of up to 4% and a 6-month mortality rate of up to 80% have been reported in those with proximal disease (thighs, abdomen wall, and buttocks). Penile calciphylaxis is very rare but has a mortality rate of 69% within 6 months. Its treatment is controversial. For small lesions, conservative treatment with local wound care and debridement may suffice. Partial or complete penectomy may be needed for more extensive lesions, and especially those associated with signs of local infection. In addition to surgical intervention, as with any other case of calcific uremic arteriolopathy, the cornerstones of therapy should be to keep serum phosphorus <6 mg/dL, and a Ca × P product <55 mg²/dL². We report here the first case of penile calciphylaxis whereby intravenous sodium thiosulfate was used in addition to the standard medical and surgical therapy. Two months after surgery, the patient's wound completely healed and he has experienced no new lesions over the past 11 months.